IMMUNOLOGY TEXTBOOK - Mercury Podillya 2013

PRIMARY IMMUNODEFICIENCY

General Principles of Immunodeficiency Management

Patients with immunodeficiencies require special attention and need not only continuous medical care, but also psychological and social support.

Diet. In the absence of malabsorption syndrome, a special diet is not required. If gastrointestinal disorders are present, a dietitian's consultation is necessary. The diet must satisfy the body's need for Proteins, Vitamins, and Trace Elements, and be sufficiently high in calories to ensure normal GROWTH AND DEVELOPMENT. Malnutrition in immunodeficiency can lead to an even greater suppression of The Immune System.

Infection Prevention is indicated for all patients with immunodeficiencies, especially in severe combined immunodeficiency.

Complete isolation of infants with severe combined immunodeficiency and keeping them in sterile incubators eliminates contact with microbes. Partial isolation is less effective, as even microorganisms non-pathogenic to healthy individuals can cause severe infections in immunodeficient patients. To reduce the risk of infection at home, the patient should Sleep in a separate bed, have their own room, and avoid contact with infected individuals, especially if the infection is caused by herpes simplex or varicella-zoster Viruses.

Immunoglobulin replacement therapy allows many patients with Humoral Immunity Deficiency to lead a normal life. The parents of a sick child are explained that the child does not need overprotection, should not avoid walks in the fresh air, and can play with other children as well as attend preschools and school.

Management of Infections

Chronic otitis media is treated with antimicrobial agents. Surgical Treatment is performed if necessary. Hearing tests are regularly conducted for the early detection and treatment of hearing loss.

Sinusitis. During exacerbations, antimicrobial and decongestant agents are prescribed. If medical treatment is ineffective, the infectious agent is identified, and the Paranasal Sinuses are drained. Other surgeries on the paranasal sinuses are rarely performed, especially in young children.

Chronic respiratory infections. Pulmonary function testing and chest radiography are performed at least once a year (more frequently if clinical deterioration occurs). In Bronchiectasis, special attention is paid to postural drainage and inhalations, which can be performed at home.

Psychosocial support is particularly necessary for patients with severe immunodeficiencies, as they experience serious psychological and financial difficulties. School teachers should be informed about the child's condition and arrange for supplementary tutoring.

Precautionary measures. When cellular immunity deficiency is suspected, whole Blood transfusions should be avoided, as donor lymphocytes can cause graft-versus-host disease. If a blood transfusion is necessary, the blood must be irradiated at a dose of 30 Gy. In addition, all blood components must be thoroughly screened for cytomegalovirus and hepatitis B and C viruses.

Live viral Vaccines, such as the oral polio vaccine, measles, mumps, and rubella vaccines, as well as the BCG vaccine, are contraindicated in immunodeficiencies. Inactivated vaccines are generally safe and can be used even for diagnostic purposes.

Tonsillectomy and adenoidectomy are performed strictly according to indications. Splenectomy is carried out only in specific cases of Wiskott-Aldrich syndrome when bleeding cannot be controlled. In all other cases, it is contraindicated because it increases the risk of severe infection.

Corticosteroids and other immunosuppressants are used very rarely.

Prophylactic administration of antimicrobials is effective in immunodeficiencies accompanied by severe infections, such as Wiskott-Aldrich syndrome. There are numerous regimens for long-term antimicrobial prophylaxis. According to one approach, multiple antimicrobial agents are prescribed concurrently, with 1-2 month intervals between courses. This regimen suppresses infection and prevents The Emergence of resistant microbial strains. Children are typically prescribed amoxicillin-clavulanate, erythromycin, and trimethoprim-sulfamethoxazole or a cephalosporin preparation, while adults receive amoxicillin-clavulanate, trimethoprim-sulfamethoxazole, and a tetracycline or cephalosporin preparation.